Double venous drainage in scimitar syndrome. Ideal anatomy for percutaneous complete cure

Abstract

Congenital venolobular or scimitar syndrome is a rare congenital cardiopulmonary anomaly consisting in a partial anomalous pulmonary venous drainage, lung hypoplasia, and anomalous systemic arterial supply to the lung. It can associate with other congenital disorders which will confer the clinical presentation and prognosis of these patients. In most of the cases, the therapeutic approach is partial, as anatomy allows only aberrant arterial embolisation. We present a 6-year-old girl with recurrent pulmonary infections, diagnosed as scimitar syndrome with double collector drainage to the inferior caval vein and left atrium, undergoing interventional catheterisation for complete correction of her disorder. The anomalous systemic artery supply was embolised and the anomalous venous drainage was occluded. The patient was asymptomatic during follow-up, which supports the interventional catheterisation approach as a valid therapeutic option in cases of scimitar syndrome with double venous drainage.